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16 Cards in this Set
- Front
- Back
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clinical features of frontotemporal dementias
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progressive language deterioration
personality changes |
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lobes affected in FTD
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frontal and temporal lobes
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aka for FTD
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tauopathy because all have accumulation of tau deposits
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FTD with Parkinsonism and tau
pathology: |
cortical atrophy, neuronal loss, gliosis, tau-tangles (4R or 3R+4R); +/- glial inclusions
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What are the tauopathies?
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FTD with Parkinsonism
Pick dz Progressive supranuclear palsy corticobasal degeneration |
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Pick dz (lobar atrophy) clinical:
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early onset behavioral change, personality change (frontal lobe), and language alterations (temporal lobe); mostly sporadic, but some familial with tau mut
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Pick dz pathology gross
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asymmetric frontal and temporal atrophy (knife-edge); SPARING of posterior two-thirds of superior temporal gyrus; essentially no parietal or occipital involvement; +/1 bilateral caudate and putamen atrophy
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Pick dz pathology micro
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severe neuronal loss in outer 3 layers of cortex, Pick cells, Pick bodies
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what are Pick cells?
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Pick cells = swelling of neurons
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what are Pick bodies?
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Pick bodies = round homogeneous cytoplasmic filamentous inclusions, weak basophilic and strongly silver stained (3R tau)
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progressive supranuclear palsy clinical
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truncal rigidity, dysequilibrium, speech and ocular disturbance, dementia
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progressive supranuclear palsy path
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globose tangles of 4R tau in globus pallidus, subthal nucl, subst nigra, periaqueductal grey, dentate
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progressive supranuclear palsy genetics
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no MAPT (tau) mutation, but some have MAPT polymorphism
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Corticobasal degeneration path
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motor cortex atrophy; neuronal loss, gliosis, "ballooned neurons" (phosphorylated neurofilaments);
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where is tau in corticobasal degeneration?
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tau (4R) is in astrocytes (tufted), oligos (coiled bodies), basal gangl neurons, cortical neurons; around astrocytes (astrocytic plaques); threads in grey and white matter; subst nigra involvement
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FTD's without tau usually contain what?
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ubiquitin inclusions
some with mut on chr 17 progranulin (close to MAPT tau) |
